肾海绵状血管瘤的诊断和治疗（附3例报告）

目的：探讨肾海绵状血管瘤的诊断与治疗。方法：回顾性分析3例肾海绵状血管瘤患者,男2例,女1例,年龄17-34岁。临床表现包括腰痛1例,肉眼血尿1例,腰痛并镜下血尿1例。多普勒超声和CT扫描显示肾脏肿块,2例体积较大,其中1例伴有肾静脉血栓形成。2例男性患者行肾切除,1例女性患者行肿块切除。结果：3例患者术后血尿,腰痛均消失。术后随访6个月-2年,无复发或并发症,复查CT和尿液检查未见异常。结论：肾海绵状血管瘤是一种罕见的良性病变,其症状主要为腰痛、血尿。肾海绵状血管瘤少有术前诊断,多为术后病检证实,CT、B超、MRI和肾血管造影均有诊断价值,但不能完全与恶性肿瘤区分。手术治疗是主要的治疗方法。     

Endovascular stent placement for the treatment of nutcracker syndrome

A patient presented with recurrent macroscopic hematuria, persistent orthostatic proteinuria and intermittent flank pain for 12 months. Doppler ultrasound, computed tomography angiography and selective renal vein phlebography revealed the compression of the left renal vein by the abdominal aorta. The patient was diagnosed with nutcracker syndrome and underwent endovascular stent placement. No intraoperative or postoperative complications occurred. The clinical symptoms completely resolved 14 days after the operation. At a follow-up of 6 months, the patient continued to be asymptomatic.     

Successful treatment of acute inferior vena cava and unilateral renal vein thrombosis by local infusion of recombinant tissue plasminogen activator

Renal vein thrombosis can occur as a complication of nephrotic syndrome. We present the case of a young man with nephrotic syndrome caused by minimal change disease who developed acute inferior vena cava and left renal vein thrombosis. He was treated initially with intravenous heparin. Because of the persistence of severe left flank pain and gross hematuria, local infusion of recombinant tissue plasminogen activator was tried, with resolution of thrombi and subsidence of symptoms. Functional preservation of the involved kidney is good, as indicated by Tc-99m DMSA scan (involved kidney, 47.4%; uninvolved kidney, 52.6%). Anticoagulation is usually recommended as the treatment of choice in renal vein thrombosis. We believe that in cases with critical presentations, such as bilateral involvement, extension into inferior vena cava, acute renal failure, pulmonary embolism or severe flank pain, thrombolytic therapy should be considered as a second-line treatment if good response is not obtained with heparin.     

Nutcracker phenomenon treated with left renal vein transposition: a case report]

Herein is reported a case of the nutcracker phenomenon with severe gross hematuria which was surgically treated. A 14-year-old boy had been suffering from left flank pain and recurrent gross hematuria causing urinary retention with blood clots. Radiological images including three-dimensional computed tomography (CT) and angiography disclosed the nutcracker phenomenon with left renal venous hypertension. Since the aforementioned symptoms could not be managed conservatively, he underwent transposition of the left renal vein at 5.5 cm caudal from the original position. Postoperative course was uneventful and the patient has had no further hematuria after the surgery.     

Spontaneous disappearance of a renal arteriovenous malformation

Abstract We describe herein a case of complete spontaneous disappearance of a congenital arteriovenous malformation (AVM). A 28‐year‐old male was hospitalized for right flank pain and gross hematuria, followed by bladder tamponade. To improve the patient's symptoms, bladder irrigation was performed. Cystoscopy demonstrated bloody urine from the right ureteral orifice. Right selective renal arteriography demonstrated tortuous, coiled vascular channels with early filling of the renal vein. Thus, right renal AVM was diagnosed. However, the patient refused further treatment and was discharged. One year later, massive hematuria recurred with bladder tamponade and the patient was rehospitalized. Renal arteriography did not show any evidence of AVM and there has been no hematuria since.     

Removal of renal cell carcinoma extending into the right atrium: report of a case]

A 65-year old man presented with a seven-month history of macrohematuria and left back pain. Abdominal ultrasonography, enhanced computed tomographic (CT) scanning, magnetic resonance imaging (MRI), selective renal angiography and vena cavography revealed a left renal tumor extending into the inferior vena cava and right atrium. Surgery was performed using the cardiopulmonary bypass and the whole tumor was resected grossly except for the tumor invading into the lumbar vein. The patient recovered promptly but died from cancer metastasis six months after operation.     

Renal leiomyoma: Case report and literature review

Renal leiomyomas are rare benign tumours of the kidney originating from muscle cells. They are usually found by an autopsy, whether the patient is asymptomatic or has symptoms (i.e., abdominal/flank pain, hematuria, palpable mass). Today the widespread use of ultrasonography and computed tomography has increased the detection of clinically asymptomatic renal leiomyomas. The differential diagnosis between leiomyomas and other malignant lesions (above all renal cell carcinoma or leiomyosarcoma) is still possible by histological examination. Radiological examinations are not sufficient for the differential diagnosis. Renal leiomyomas have no aggressive behaviour and they usually do not metastasize. The prognosis, after surgery, is excellent without recurrence. We report a case of leiomyoma in a 31-year-old man who presented hematuria and flank pain. We also review the literature and provide a summary of clinical, radiological and histological features of renal leiomyomas.     

Renal vein thrombosis.

The manifestations, clinical course and treatment of 14 patients with non-malignant renal vein thrombosis are described. Most patients (10 of 14) had generalized vague illness and nephrotic syndrome but 4 were initially seen with acute symptoms of flank pain, hematuria or hypertension. Renal vein thrombosis affected young men 2.5 times more often than women and occurred on the left side 2.6 times more commonly than on the right or both sides. Red blood cell casts in the urinary sediment, heavy proteinuria and hypoalbuminemia were useful indicators of the disease. Excretory urographic signs were suggestive of renal vein thrombosis in all patients and these were corroborated by angiographic studies. Systemic anticoagulation with or without a renal failure program and diuretics, or simply a combination of the last 2 modalities, was used in 9 patients. In 2 of the 9 patients who were unresponsive the adjuvant use of cyclophosphamide and steroids effected a cure. The remaining 5 patients underwent nephrectomy or thrombectomy. All 14 patients were followed for 1 to 7 years (mean 1.6 years). Ten patients were cured or improved, 1 patient was unchanged, and in the remaining 3 patients the condition deteriorated and they subsequently required a renal allograft. The rationale for various forms of treatment is discussed.     

Arteriovenous fistaul from rupture of abdominal aortic aneurysm.

Two patients with spontaneous aortocaval fistulas are described and compared with 67 cases reported in the English literature. Symptoms may vary widely; however, the presence of an expansile abdominal mass with a continuous bruit is usually diagnostic. The only successful management is promt repair of the vena caval defect and the aortic aneurysm. A third patient with spontaneous rupture of an abdominal aortic aneurysm into the left renal vein is alos described. Only five similar cases could be found in the work literature. Left flank pain, pulsatile abdominal mass, continuous bruit, and hematuria is the usual clinical picture. All of these cases involved an anomalous left retroaortic renal vein and all patients survived the necessary surgical correction. The operation of choice is closure of the defect in the retroaortic left renal vein and repair of the aneurysm.     

Transposition of the left renal vein in nutcracker syndrome.

Mesoaortic compression of left renal vein produces left renal vein hypertension resulting in left flank pain, hematuria and pelvic-ureteral varices. This is called the nutcracker syndrome. The nutcracker syndrome has been treated in various ways. We recently experienced two cases of patients with nutcracker syndrome. We treated the patients with transposition of their left renal vein.     

Renal medullary carcinoma. Case report and review of the literature

Young patients with sickle cell trait or disease present a higher risk for renal medullary carcinoma, an aggressive renal tumor, with dismal prognosis, with a median survival of 4 months from the time of diagnosis. We report a 26-year-old patient with a 2-month history of bone pain at the right iliac crest, loss of weight, recurrent macroscopic hematuria and abdominal mass. Imaging studies demonstrated a 10-cm mass in the right kidney. Pathological evaluation revealed a medullary carcinoma and the patient died after 4 months, despite adjuvant treatment. Whenever a patient with sickle cell trait or disease presents with hematuria and/or flank pain, the possibility of a renal medullary carcinoma should always be kept in mind, with thorough investigation and expeditious treatment.     

A case with arteriovenous fistula from renal injury]

We report on a 58-year-old male treated with transcatheter embolization for arteriovenous fistula due to renal injury. The patient was transferred to our hospital on December 10, 1989, with left renal injury and left temporal bone fractures. Enhanced CT revealed parenchymal fracture with peri-renal hematoma in the left kidney. Antibiotics were prescribed to prevent bacterial infection of the injured kidney. Urine cleared and pain in the left loin area disappeared 17 days later. However, on December 28, gross hematuria and pain in the left flank suddenly reappeared. He was given a blood transfusion and was diagnosed with re-bleeding from the injured kidney. Renal angiography performed on January 10, 1990, revealed arteriovenous fistula of the kidney. Transcatheter embolization was done using a metal coil to close the arteriovenous fistula. The patient has had no further problems or complaints. Excretory pyelogram showed no hydronephrosis in the left kidney. TcDMSA renoscintiscan showed a defect in the mid-portion of the kidney. These results suggest that transcatheter embolization can be useful to alleviate arteriovenous fistula in cases of renal injury.     

The role of imaging techniques in diagnostic evaluation of angiomyolipomas.

Renal AML are rare benign tumors of the kidneys consisting of fat tissue intermixed with thick-walled blood vessels and smooth muscle. Due to the easy access to modern imaging techniques more and more AML are detected incidentally during diagnostic evaluation of common urological disease before getting symptomatic (mostly flank pain, hematuria). The presence of a highly echodense renal mass on ultrasound and the detection of even small amounts of fat in CT usually allows to establish the diagnosis of AML. If these procedures give still equivocal results, angiography and MRI may become necessary. When report a case of a patient with AML where all the imaging techniques including magnetic resonance were nonconclusive. The correct diagnosis could only be established by histological examination after surgical resection. Indeed, the presence of a large hematoma had masked all the characteristic features of AML in this case.     

Local thrombolytic treatment for renal arterial embolism

OBJECTIVE: To determine the utility of local thrombolysis in the treatment of acute renal arterial occlusion. METHODS: We used local thrombolytic treatment in a female patient, aged 76, with 72 h of anuria, right lumbar and flank pain. She had a 3-year history of ischemic heart disease and atrial fibrillation controlled with digital treatment. Also, she was nephrectomized on the left side 33 years ago for lithiasic pyonephrosis. A normal right urinary tract was demonstrated with ultrasound examination, KUB radiography and retrograde pyelography. The next step was diagnostic abdominal angiography and local thrombolytic treatment with streptokinase. RESULT: Thrombolysis with streptokinase was successful following 72 h of renal artery occlusion. After 24 months the patient is doing well. CONCLUSION: Local intra-arterial thrombolysis is the treatment of choice in renal artery occlusion.     

Primary renal angiosarcoma

Primary renal Angiosarcoma is a rare neoplasm and only 24 cases have been reported in specialized literature. We describe a case of primary renal angiosarcoma in a patient presenting with hematuria, palpable abdominal mass, left flank pain and anemia. A computerized tomography of the abdomen with contrast medium showed a tumor with 15 cm diameter, in the upper pole of the left kidney, with a low-density central area, suggesting necrosis or hemorrhage. Diagnosis was given in a morphologic base and proven by an immunohistochemical study. Primary renal angiosarcoma should be included among differential diagnosis of retroperitoneal hematoma and hemorrhagic renal tumors.     

Autosomal dominant polycystic kidney disease: presentation, complications, and prognosis

Fifty-three symptomatic adults with autosomal dominant polycystic kidney disease were studied retrospectively for a mean follow-up of 12 years (range 10 months to 33 years). Diagnosis was confirmed by either x-ray, ultrasound, laparotomy, or autopsy. Commonest presenting clinical findings were flank pain (30%), hypertension (21%), symptomatic urinary tract infection (UTI) (19%), gross hematuria (19%), and palpable masses (15%). A total of nine patients (17%) progressed to end-stage renal disease. Change in renal function measured using the reciprocal of plasma creatinine plotted against time was linear for each individual patient with a maximum functional decline of 0.7 mg/dL/yr (slope = -0.07). Past the age of sixty renal failure was uncommon. Easily controlled hypertension developed in 64% attended by mild retinopathy. UTIs were common (53%), often recurrent (61%), precipitated by instrumentation in 6 of 14 patients (43%), leading to death in two (33%). Renal calculi were extremely common (34%) and had no defined metabolic cause. The presence of hematuria (64%), gross or microscopic, bore no relationship to the decline in renal function. Pregnancy was normal in these patients with no increase in fetal or maternal morbidity or mortality. We conclude the following: Renal functional deterioration is linear, less than previously reported, and bears no relationship to hematuria. Hypertension is common, easily treated, and causes minor end-organ damage. Renal calculi are frequent. Urinary tract instrumentation often induces infection with considerable morbidity and mortality and must be avoided. Pregnancy is not contraindicated if renal function is normal. The prognosis for survival in this disease is better than previously reported.     

Recidive of renal cell carcinoma tumor thrombus in inferior vena cava via lumbar vein

Renal cell carcinoma (RCC) develops tumor thrombus in the renal vein and inferior vena cava (IVC) in 10% of cases. Surgical treatment is radical nephrectomy and thrombectomy of the IVC. Local recidive can develop in the lumbar fossa, lymph nodes, and the IVC. We report a 58-year-old patient admitted to the Clinic for Urology at the Military Medical Academy, Belgrade, Serbia, in February 2009 with RCC of the left kidney and tumor thrombus in the IVC. After ultrasonography exam and multislice computed tomography scan, we performed radical nephrectomy and thrombectomy of the IVC (level II). Four months after the operation, ultrasound exam and cavography showed intracaval and paracaval recidive tumor masses in the renal part of the IVC. On operation we removed intraluminal IVC thrombus, which arises from the lumbar vein on the IVC posterior wall, with paracaval thrombus in the lumbar vein. We conclude that RCC tumor thrombus can spread from the kidney to the IVC through the lumbar vein.     

Spectrum of renal vascular malformation.

In summary, renal AVMs are rare. The possibility of these lesions should be raised in cases of hematuria of unknown etiology, prolonged hematuria following trauma or needle biopsy, unexplained congestive heart failure or abdominal/flank bruit, or hypertension after renal trauma. Selective renal arteriography is the study of choice for establishing the diagnosis, the hallmark being demonstration of an abnormal arterial communication with a vein. The goal of management should be maximum preservation of renal parenchyma. Observation with follow-up is indicated for asymptomatic AVMs.     

Left renal venous hypertension “nutcracker” syndrome: Managed by Direct Renocaval Reimplantation

Since 1978 a diagnosis of left renal venous hypertension has been established in 3 patients. All 3 had otherwise unexplained left flank and abdominal pain, 2 had left renal gross hematuria, and 2 were found to have ureteral and peripelvic varices demonstrated by selective renal venography. All were found to have mild to moderate pressure gradients between the left renal vein and vena cava, presumably due to compression of the left renal vein between the aorta and overlying superior mesenteric artery. Direct left renocaval reimplantation was performed in all cases, with resection of the collateral varices that were present in 2 cases. Severe unrelenting pain was the primary indication for surgery in all cases, rather than hematuria. All remain asymptomatic one to four years after surgery.     

Acute renal failure, associated with non-steroidal anti-inflammatory drugs in healthy children

Seven patients aged 13 to 17.5 years developed acute renal failure after treatment with a variety of non-steroidal anti-inflammatory drugs (NSAID): naproxen, diclofenac, ibuprofen, dipyrone and paracetamol. Six of the patients used more than one kind of NSAID. None of the patients had previous history of renal disease or concomitant treatment with other drugs. The time interval between NSAID administration to the emergence of symptoms ranged from 1 to 4 days. The most common presenting symptoms were flank pain (4 patients), abdominal pain (3 patients) and vomiting (3 patients). All patients had normal urine output. Microscopic hematuria and proteinuria were found in 5 patients and leukocyturia in 2. Serum creatinine ranged from 1.3 to 8.3 mg% at presentation. Kidney biopsy was performed in 3 patients and showed findings consistent with mild interstitial inflammation in 1 patient, and normal renal tissue in 2. All patients were treated with intravenous fluids, 1 received corticosteroids. Renal function completely normalized in all patients within 7 to 16 days.